Materiały źródłowe

• #1 Paraganglioma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/paraganglioma/diagnosis-treatment/drc-20575710

  Most often, paraganglioma treatment involves surgery to remove the tumor. If the tumor makes hormones, healthcare professionals often use medicines to block the hormones first. If the paraganglioma can’t be removed with surgery or if it spreads, you may need other treatments. […] Your treatment options for paraganglioma depend on various factors. These include: Where the tumor is located. Whether it is cancerous and has spread to other parts of the body. Whether it makes extra hormones that cause symptoms. […] Treatment options include: […] If your paraganglioma makes extra catecholamines, you’ll likely need treatments to block the effects or lower the levels of these hormones. These treatments lower high blood pressure and control other symptoms. It’s important for blood pressure and symptoms to be under control before other paraganglioma treatment starts. That’s because treatment can cause the tumor to release very large amounts of catecholamines and that can lead to serious problems.

• #2 Paraganglioma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22394-paraganglioma

  How is paraganglioma treated? Treatment options for paraganglioma depend on several factors, including: […] If you have a paraganglioma that causes symptoms due to excess adrenal hormones, your healthcare provider will likely recommend medication to manage the symptoms. Medications may include: […] Treatment options for paraganglioma include: […] Surgery is the main form of treatment for paragangliomas. During the surgery to remove the tumor, your surgeon will check the surrounding tissue and lymph nodes to see if the tumor has spread. […] Radiation therapy is a cancer treatment that focuses strong beams of energy to destroy cancer cells or keep them from growing while sparing as much surrounding healthy tissue as possible. […] Chemotherapy is the standard therapy for treating metastatic paraganglioma. […] Ablation therapy is a minimally invasive treatment option that uses very high or very low temperatures to destroy tumors. […] Targeted therapy is a treatment option that uses medications or other substances to attack specific cancer cells without harming healthy cells.

• #3 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  Paragangliomas, if detected early, can be successfully treated and managed in the vast majority of cases. If possible, the treatment of choice for the condition is surgery to remove the tumor(s), but there are other treatment options. Surgical treatment usually alleviates symptoms. Once diagnosed, it is recommended to be seen by a multi-disciplinary medical team with pheo para experience. […] When surgery is not an option due to multiple tumor sites, metastatic disease, or the location of the paraganglioma, chemotherapy, radiation or radiopharmaceutical therapy are also treatment options. […] Surgery to remove a paraganglioma is the preferred treatment option. During surgery, the tissue and lymph nodes close to the tumor may be checked and if the tumor has spread, these tissues may also be removed. Drugs may be given before, during, and after surgery to keep blood pressure and heart rate normal.

• #4 Pheochromocytoma and Paraganglioma: From Treatment to Follow-up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7751245/

  Pheochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors arising from chromaffin cells in the adrenal medulla, sympathetic or parasympathetic ganglia. Currently, the only curative treatment option of pheochromocytomas/paraganglioma (PPGL) is surgical resection. Surgery aims to eliminate both risks of hypersecretion and tumor growth. The consequences of hypersecretion should be carefully controlled with medical therapy before and during the surgery. […] Therefore, all patients with PPGL who are surgically treated should be followed for at least 10 years for recurrent disease and new tumor formation. […] Although surgical resection is the only curative treatment for PPGLs, surgical treatment is palliative except for resectable locoregional metastases in metastatic disease or for isolated distant metastases. The purpose of palliative treatment is to reduce hormone secretion and prevent metastasis-related complications in a critical anatomical location.

• #5 Paraganglioma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/paraganglioma/diagnosis-treatment/drc-20575710

  Most often, paraganglioma treatment involves surgery to remove the tumor. If the tumor makes hormones, healthcare professionals often use medicines to block the hormones first. If the paraganglioma can’t be removed with surgery or if it spreads, you may need other treatments. […] Your treatment options for paraganglioma depend on various factors. These include: Where the tumor is located. Whether it is cancerous and has spread to other parts of the body. Whether it makes extra hormones that cause symptoms. […] Treatment options include: […] If your paraganglioma makes extra catecholamines, you’ll likely need treatments to block the effects or lower the levels of these hormones. These treatments lower high blood pressure and control other symptoms. It’s important for blood pressure and symptoms to be under control before other paraganglioma treatment starts. That’s because treatment can cause the tumor to release very large amounts of catecholamines and that can lead to serious problems.

• #6 Pheochromocytoma and Paraganglioma: From Treatment to Follow-up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7751245/

  Pheochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors arising from chromaffin cells in the adrenal medulla, sympathetic or parasympathetic ganglia. Currently, the only curative treatment option of pheochromocytomas/paraganglioma (PPGL) is surgical resection. Surgery aims to eliminate both risks of hypersecretion and tumor growth. The consequences of hypersecretion should be carefully controlled with medical therapy before and during the surgery. […] Therefore, all patients with PPGL who are surgically treated should be followed for at least 10 years for recurrent disease and new tumor formation. […] Although surgical resection is the only curative treatment for PPGLs, surgical treatment is palliative except for resectable locoregional metastases in metastatic disease or for isolated distant metastases. The purpose of palliative treatment is to reduce hormone secretion and prevent metastasis-related complications in a critical anatomical location.

• #7 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  Bisphosphonate or denosumab therapy should be considered in the case of bone metastases by analogy with other types of neuroendocrine tumors. […] Adequate blood pressure control with alpha adrenoceptor blockade at least 10–14 days prior to surgery is essential in functioning PCCs/PGLs to prevent severe cardiovascular events during surgery. […] In palliative scenarios, alpha adrenoceptor blockade should also be considered—balanced against side effects—to alleviate hormonal symptoms and prevent complications from catecholamine excess. […] It has been conventional to use phenoxybenzamine at starting doses approximating 10 mg 2–3x per day, although other similar drugs such as doxazosin and prazosin have been used. […] There is no clear evidence for the superiority of one alpha-blocker for the pre-operative blockade of PCC/PGL patients, as previously reviewed.

• #8 Paraganglioma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26614

  Radiation therapy is reserved for patients with small, non-secretory, benign tumors in the skull base and neck when removal would involve damage to critical vascular or nerve structures. It is also considered appropriate for patients with jugular paragangliomas and more advanced tympanic paragangliomas unless they are symptomatic from catecholamine secretion or tumor bulk. Radiation therapy is also helpful with recurrent tumors after surgical resection. […] Preoperative preparation is necessary to prevent hypertensive crisis, malignant arrhythmias, and multiorgan failure intraoperatively. Clinicians typically initiate antihypertensive medications at least 7 days before surgery to achieve low-normal blood pressure, especially in patients with catecholamine-secreting tumors. […] Patients experiencing recurrent disease after surgical resection may consider radiotherapy or repeat resection. A retrospective review of recurrent head and neck paragangliomas treated with radiotherapy or surgery demonstrated 5-year freedom from 100% and 62% progression, respectively.

• #9 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  Bisphosphonate or denosumab therapy should be considered in the case of bone metastases by analogy with other types of neuroendocrine tumors. […] Adequate blood pressure control with alpha adrenoceptor blockade at least 10–14 days prior to surgery is essential in functioning PCCs/PGLs to prevent severe cardiovascular events during surgery. […] In palliative scenarios, alpha adrenoceptor blockade should also be considered—balanced against side effects—to alleviate hormonal symptoms and prevent complications from catecholamine excess. […] It has been conventional to use phenoxybenzamine at starting doses approximating 10 mg 2–3x per day, although other similar drugs such as doxazosin and prazosin have been used. […] There is no clear evidence for the superiority of one alpha-blocker for the pre-operative blockade of PCC/PGL patients, as previously reviewed.

• #10 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  Nevertheless, perioperative hypertension seems to be slightly better controlled with phenoxybenzamine (especially in those patients with high catecholamine or metanephrine levels), although with more pronounced postoperative hypotension. […] Indeed, there were fewer side effects in the doxazosin group. […] Moreover, in functioning metastatic PCCs/PGLs, pre-treatment alpha blockade is recommended prior to initiation of therapy to prevent symptomatic catecholamine release in response to locoregional or systemic treatment. […] Furthermore, it is important to mention that beta adrenoceptor blockers must not be given prior to initiation of an adequate alpha adrenoceptor blockade. […] For metastatic PCCs/PGLs, there are few established treatment options but radiotherapy ([131I]MIBG therapy, recently PRRT) as well as classic chemotherapy (Averbuch scheme, and temozolomide), and different targeted therapy options, have been extensively used outside of controlled clinical trials.

• #11 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  Bisphosphonate or denosumab therapy should be considered in the case of bone metastases by analogy with other types of neuroendocrine tumors. […] Adequate blood pressure control with alpha adrenoceptor blockade at least 10–14 days prior to surgery is essential in functioning PCCs/PGLs to prevent severe cardiovascular events during surgery. […] In palliative scenarios, alpha adrenoceptor blockade should also be considered—balanced against side effects—to alleviate hormonal symptoms and prevent complications from catecholamine excess. […] It has been conventional to use phenoxybenzamine at starting doses approximating 10 mg 2–3x per day, although other similar drugs such as doxazosin and prazosin have been used. […] There is no clear evidence for the superiority of one alpha-blocker for the pre-operative blockade of PCC/PGL patients, as previously reviewed.

• #12 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  Nevertheless, perioperative hypertension seems to be slightly better controlled with phenoxybenzamine (especially in those patients with high catecholamine or metanephrine levels), although with more pronounced postoperative hypotension. […] Indeed, there were fewer side effects in the doxazosin group. […] Moreover, in functioning metastatic PCCs/PGLs, pre-treatment alpha blockade is recommended prior to initiation of therapy to prevent symptomatic catecholamine release in response to locoregional or systemic treatment. […] Furthermore, it is important to mention that beta adrenoceptor blockers must not be given prior to initiation of an adequate alpha adrenoceptor blockade. […] For metastatic PCCs/PGLs, there are few established treatment options but radiotherapy ([131I]MIBG therapy, recently PRRT) as well as classic chemotherapy (Averbuch scheme, and temozolomide), and different targeted therapy options, have been extensively used outside of controlled clinical trials.

• #13 Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Skin Center

  https://www.skincenter.com/patient-education/healthwise?DOCHWID=ncicdr0000681246

  Phenoxybenzamine use is safe in pregnancy, but beta-adrenergic blockers should be initiated only if needed because their use has been associated with intrauterine growth restriction. […] Resection of the tumor can often be performed safely during the second trimester, or tumor resection can be combined with cesarean delivery for patients diagnosed later in pregnancy.

• #14 Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Skin Center

  https://www.skincenter.com/patient-education/healthwise?DOCHWID=ncicdr0000681246

  Phenoxybenzamine use is safe in pregnancy, but beta-adrenergic blockers should be initiated only if needed because their use has been associated with intrauterine growth restriction. […] Resection of the tumor can often be performed safely during the second trimester, or tumor resection can be combined with cesarean delivery for patients diagnosed later in pregnancy.

• #15 Pheochromocytoma and Paraganglioma: From Treatment to Follow-up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7751245/

  Pheochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors arising from chromaffin cells in the adrenal medulla, sympathetic or parasympathetic ganglia. Currently, the only curative treatment option of pheochromocytomas/paraganglioma (PPGL) is surgical resection. Surgery aims to eliminate both risks of hypersecretion and tumor growth. The consequences of hypersecretion should be carefully controlled with medical therapy before and during the surgery. […] Therefore, all patients with PPGL who are surgically treated should be followed for at least 10 years for recurrent disease and new tumor formation. […] Although surgical resection is the only curative treatment for PPGLs, surgical treatment is palliative except for resectable locoregional metastases in metastatic disease or for isolated distant metastases. The purpose of palliative treatment is to reduce hormone secretion and prevent metastasis-related complications in a critical anatomical location.

• #16 Pheochromocytoma and Paraganglioma: From Treatment to Follow-up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7751245/

  The main treatment of PPGL is surgery, and it offers the best chance for cure or remission. According to the patients biochemical tests, genetic tests, anatomical and functional imaging results, the surgery is planned individually. […] Laparoscopic adrenalectomy is recommended for the treatment of many patients with PCC. […] While laparoscopic adrenalectomy is recommended in PCCs up to 6 cm, open adrenalectomy is recommended to provide complete tumor resection, prevent tumor rupture and local recurrence in PCCs over 6 cm. Open surgery is recommended in PGLs because they have a higher risk of malignancy, and the tumor is often located in difficult anatomical regions for laparoscopic resection. […] Although surgical resection is the only curative treatment for PPGLs, surgical treatment is palliative except for resectable locoregional metastases in metastatic disease or for isolated distant metastases. The purpose of palliative treatment is to reduce hormone secretion and prevent metastasis-related complications in a critical anatomical location.

• #17 Pheochromocytoma and Paraganglioma: From Treatment to Follow-up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7751245/

  The main treatment of PPGL is surgery, and it offers the best chance for cure or remission. According to the patients biochemical tests, genetic tests, anatomical and functional imaging results, the surgery is planned individually. […] Laparoscopic adrenalectomy is recommended for the treatment of many patients with PCC. […] While laparoscopic adrenalectomy is recommended in PCCs up to 6 cm, open adrenalectomy is recommended to provide complete tumor resection, prevent tumor rupture and local recurrence in PCCs over 6 cm. Open surgery is recommended in PGLs because they have a higher risk of malignancy, and the tumor is often located in difficult anatomical regions for laparoscopic resection. […] Although surgical resection is the only curative treatment for PPGLs, surgical treatment is palliative except for resectable locoregional metastases in metastatic disease or for isolated distant metastases. The purpose of palliative treatment is to reduce hormone secretion and prevent metastasis-related complications in a critical anatomical location.

• #18 Pheochromocytoma and Paraganglioma: From Treatment to Follow-up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7751245/

  The main treatment of PPGL is surgery, and it offers the best chance for cure or remission. According to the patients biochemical tests, genetic tests, anatomical and functional imaging results, the surgery is planned individually. […] Laparoscopic adrenalectomy is recommended for the treatment of many patients with PCC. […] While laparoscopic adrenalectomy is recommended in PCCs up to 6 cm, open adrenalectomy is recommended to provide complete tumor resection, prevent tumor rupture and local recurrence in PCCs over 6 cm. Open surgery is recommended in PGLs because they have a higher risk of malignancy, and the tumor is often located in difficult anatomical regions for laparoscopic resection. […] Although surgical resection is the only curative treatment for PPGLs, surgical treatment is palliative except for resectable locoregional metastases in metastatic disease or for isolated distant metastases. The purpose of palliative treatment is to reduce hormone secretion and prevent metastasis-related complications in a critical anatomical location.

• #19 Pheochromocytoma and Paraganglioma Symptoms and Treatment | Saint John’s Cancer Institute

  https://www.saintjohnscancer.org/endocrine/conditions/pheochromocytoma-and-paraganglioma/

  Pheochromocytoma Treatment (and PPGL) […] The only treatment for a pheochromocytoma or paraganglioma is surgery to remove your adrenal gland (which includes your adrenal nodule), which should be performed by a specialized adrenal, endocrine surgeon. […] For a pheochromocytoma, this involves an adrenalectomy, usually done via a minimally invasive, laparoscopic approach. However, if there is concern for, or a diagnosis of cancer, an open adrenalectomy may be performed. For patients with certain genetic mutations, occasionally a partial adrenalectomy may be offered. […] The majority of paragangliomas are removed with open surgery, though occasionally a minimally invasive laparoscopic technique can be used. […] All patients with functional, hormone-producing paragangliomas and pheochromocytomas should be started on an alpha-blocker 7-14 days before surgery.

• #20 Pheochromocytoma and Paraganglioma: From Treatment to Follow-up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7751245/

  The main treatment of PPGL is surgery, and it offers the best chance for cure or remission. According to the patients biochemical tests, genetic tests, anatomical and functional imaging results, the surgery is planned individually. […] Laparoscopic adrenalectomy is recommended for the treatment of many patients with PCC. […] While laparoscopic adrenalectomy is recommended in PCCs up to 6 cm, open adrenalectomy is recommended to provide complete tumor resection, prevent tumor rupture and local recurrence in PCCs over 6 cm. Open surgery is recommended in PGLs because they have a higher risk of malignancy, and the tumor is often located in difficult anatomical regions for laparoscopic resection. […] Although surgical resection is the only curative treatment for PPGLs, surgical treatment is palliative except for resectable locoregional metastases in metastatic disease or for isolated distant metastases. The purpose of palliative treatment is to reduce hormone secretion and prevent metastasis-related complications in a critical anatomical location.

• #21 Pheochromocytoma and Paraganglioma: From Treatment to Follow-up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7751245/

  The main treatment of PPGL is surgery, and it offers the best chance for cure or remission. According to the patients biochemical tests, genetic tests, anatomical and functional imaging results, the surgery is planned individually. […] Laparoscopic adrenalectomy is recommended for the treatment of many patients with PCC. […] While laparoscopic adrenalectomy is recommended in PCCs up to 6 cm, open adrenalectomy is recommended to provide complete tumor resection, prevent tumor rupture and local recurrence in PCCs over 6 cm. Open surgery is recommended in PGLs because they have a higher risk of malignancy, and the tumor is often located in difficult anatomical regions for laparoscopic resection. […] Although surgical resection is the only curative treatment for PPGLs, surgical treatment is palliative except for resectable locoregional metastases in metastatic disease or for isolated distant metastases. The purpose of palliative treatment is to reduce hormone secretion and prevent metastasis-related complications in a critical anatomical location.

• #22 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  Paragangliomas, if detected early, can be successfully treated and managed in the vast majority of cases. If possible, the treatment of choice for the condition is surgery to remove the tumor(s), but there are other treatment options. Surgical treatment usually alleviates symptoms. Once diagnosed, it is recommended to be seen by a multi-disciplinary medical team with pheo para experience. […] When surgery is not an option due to multiple tumor sites, metastatic disease, or the location of the paraganglioma, chemotherapy, radiation or radiopharmaceutical therapy are also treatment options. […] Surgery to remove a paraganglioma is the preferred treatment option. During surgery, the tissue and lymph nodes close to the tumor may be checked and if the tumor has spread, these tissues may also be removed. Drugs may be given before, during, and after surgery to keep blood pressure and heart rate normal.

• #23

  https://www.cancerhelpessentiahealth.org/Cancer_Types/pheochromocytoma_277407E1_05.html

  Treatment of localized benign pheochromocytoma or paraganglioma is usually surgery to completely remove the tumor. If the tumor is in the adrenal gland, the entire adrenal gland is removed. […] Treatment for inherited pheochromocytoma that forms in one adrenal gland is surgery to completely remove the gland. This surgery may help patients avoid lifelong steroid hormone replacement therapy and acute adrenal insufficiency. […] Treatment of pheochromocytoma or paraganglioma that has spread to nearby organs or lymph nodes is surgery to completely remove the tumor. Nearby organs that the cancer has spread to, such as the kidney, liver, part of a major blood vessel, and lymph nodes, may also be removed. […] Treatment of metastatic pheochromocytoma or paraganglioma may include the following: Surgery to completely remove the cancer, including tumors that have spread to distant parts of the body. […] Treatment of recurrent pheochromocytoma or paraganglioma may include the following: Surgery to completely remove the cancer.

• #24 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  Surgery is always the therapy of choice of non-metastatic PCC/PGL, whenever possible. However, surgery of non-functioning HN PGLs has to be carefully balanced against surgery-related morbidities, especially for the cranial nerves for vagal and jugular PGLs. In cases of a high risk related to surgery, radiotherapy/radiosurgery (gamma-knife/cyberknife) might be a less invasive option with non-curative but controlling outcomes. […] In patients with hereditary PCCs, cortical sparing surgery should always be considered since there is frequently a high risk of bilateral PCCs in hereditary disease, and cortical-sparing surgery was not associated with decreased survival despite PCC recurrence in 13% of cases in a very recently published study. […] With metastatic disease, primary tumor resection should be recommended if feasible in order to alleviate cardiovascular and other symptoms from catecholamine excess or from tumor invasion, and to minimize the target for radiopharmaceutical therapies. Moreover, several studies have shown that surgical resection of the primary tumor is associated with improved survival even with metastatic disease.

• #25 Progress in the diagnosis and treatment of paraganglioma – Wang – Translational Cancer Research

  https://tcr.amegroups.org/article/view/32832/html

  Surgery is the main treatment for PGL and can be curative for primary, recurrent, and localized metastatic tumors. […] Laparoscopic surgery can achieve the same therapeutic effect as open surgery and was even better in a recent retrospective study comparing the efficacy of laparoscopic and open surgery in metastatic PPGL, with a 44.4% cure rate for laparoscopic surgery compared to 29.3% for open surgery. […] It is important that blood pressure is controlled in patients with hypertension prior to surgery. […] According to the guidelines of the Endocrine Society, antihypertensive expansion therapy is required at least 714 days before surgery. […] Although preoperative blood pressure lowering and expansion therapy can prevent blood pressure fluctuations caused by rapid increases in catecholamine load during surgery, continuous intraoperative monitoring of blood pressure by arterial catheter is still required, to help the anesthesiologists control blood pressure.

• #26 Management of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement | Nature Reviews Endocrinology

  https://www.nature.com/articles/s41574-023-00926-0

  We recommend that cortex-sparing resection should not be offered for SDHB phaeochromocytomas due to an increased risk of local recurrence and/or metastasis (Grade 1, low). […] We recommend that patients with SDHB primary PPGL with local invasion, debilitating catecholamine excess or mass effects on adjacent organs or structures should be evaluated for tumour resection with multidisciplinary planning, especially if the tumour is affecting quality of life (Grade 1, very low). […] We recommend adrenoceptor blockers for the treatment of catecholamine-associated manifestations associated with SDHB PPGLs (Grade 1, low). […] We recommend that targeted radionuclide therapy with iodine-131 meta-iodobenzylguanidine ([131I]MIBG) or peptide receptor radionuclide therapy (PRRT) should be considered as a first-line treatment in patients with inoperable SDHB PPGL if there is slow-to-moderate progression with moderate-to-high tumour burden (Grade 1, low).

• #27 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  Surgery is always the therapy of choice of non-metastatic PCC/PGL, whenever possible. However, surgery of non-functioning HN PGLs has to be carefully balanced against surgery-related morbidities, especially for the cranial nerves for vagal and jugular PGLs. In cases of a high risk related to surgery, radiotherapy/radiosurgery (gamma-knife/cyberknife) might be a less invasive option with non-curative but controlling outcomes. […] In patients with hereditary PCCs, cortical sparing surgery should always be considered since there is frequently a high risk of bilateral PCCs in hereditary disease, and cortical-sparing surgery was not associated with decreased survival despite PCC recurrence in 13% of cases in a very recently published study. […] With metastatic disease, primary tumor resection should be recommended if feasible in order to alleviate cardiovascular and other symptoms from catecholamine excess or from tumor invasion, and to minimize the target for radiopharmaceutical therapies. Moreover, several studies have shown that surgical resection of the primary tumor is associated with improved survival even with metastatic disease.

• #28 Carotid Body Tumors Treatment & Management: Approach Considerations, Surgical Therapy, Preoperative Details

  https://emedicine.medscape.com/article/1575155-treatment

  Nonetheless, surgical treatment of carotid body tumors can be associated with significant morbidities, especially with large Shamblin III tumors (greater than 5 cm). […] For this reason, patients who have poor medical conditions, elderly patients, or patients with recurrent tumors are usually treated with radiation therapy. […] Preoperative evaluation is extremely important to avoid major surgical complications. […] Preoperative embolization is still controversial, although it has been used to decrease the risk of intraoperative bleeding, particularly in larger tumors. […] A study by Cobb et al indicated that presurgical tumor embolization provides no benefit in patients undergoing carotid body tumor (CBT) resection, finding no significant differences in mortality, cranial nerve injury, and blood loss between patients who underwent embolization and those who did not.

• #29 Carotid Body Tumors Treatment & Management: Approach Considerations, Surgical Therapy, Preoperative Details

  https://emedicine.medscape.com/article/1575155-treatment

  In contrast, a literature review by Texakalidis et al reported that in patients who underwent embolization prior to resection, intraoperative blood loss was significantly lower and operative time shorter than in those who did not. […] A study by Zhang et al indicated that preoperative tumor embolization, carried out less than 48 hours before surgery, can significantly reduce blood loss, surgical time, and length of hospital stay in patients undergoing carotid body tumor resection. […] A study by Cobb et al also indicated that preoperative arterial embolization does not contribute to the safety of carotid body tumor resection.

• #30 Paraganglioma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/paraganglioma/diagnosis-treatment/drc-20575710

  Surgery can be done to remove a paraganglioma. Even if a paraganglioma can’t be fully removed, your healthcare professional may recommend surgery to remove as much of the tumor as possible. […] Radiation therapy uses powerful energy beams to treat tumors. The energy can come from X-rays, protons or other sources. Your healthcare professional may recommend radiation if your paraganglioma can’t be removed completely with surgery. Radiation also can help ease pain caused by a paraganglioma that spreads to other parts of the body. […] Ablation therapy uses heat or cold to kill tumor cells and control the growth of the paraganglioma. This may be an option if a paraganglioma has spread to other parts of the body. […] Chemotherapy is a treatment that uses strong medicines. If your paraganglioma has spread, your healthcare professional may recommend chemotherapy to help shrink the tumors. If your paraganglioma makes extra hormones, you receive medicines to control the hormone levels before starting chemotherapy.

• #31 Paraganglioma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/paraganglioma/diagnosis-treatment/drc-20575710

  Surgery can be done to remove a paraganglioma. Even if a paraganglioma can’t be fully removed, your healthcare professional may recommend surgery to remove as much of the tumor as possible. […] Radiation therapy uses powerful energy beams to treat tumors. The energy can come from X-rays, protons or other sources. Your healthcare professional may recommend radiation if your paraganglioma can’t be removed completely with surgery. Radiation also can help ease pain caused by a paraganglioma that spreads to other parts of the body. […] Ablation therapy uses heat or cold to kill tumor cells and control the growth of the paraganglioma. This may be an option if a paraganglioma has spread to other parts of the body. […] Chemotherapy is a treatment that uses strong medicines. If your paraganglioma has spread, your healthcare professional may recommend chemotherapy to help shrink the tumors. If your paraganglioma makes extra hormones, you receive medicines to control the hormone levels before starting chemotherapy.

• #32 Progress in the diagnosis and treatment of paraganglioma – Wang – Translational Cancer Research

  https://tcr.amegroups.org/article/view/32832/html

  Since the beginning of the 21st century, extracorporeal radiation (EBRT) has been increasingly used in PGL treatment. […] For patients with advanced or unresectable metastatic PGL, radiation therapy can effectively improve symptoms and control tumor growth. […] Interventional radiology techniques for local non-surgical treatment of metastatic PGL include radiofrequency ablation (RFA), cryoablation (CRYO), and percutaneous ethanol injection (PEI). […] For patients with nonresectable, quickly progressing metastatic PPGL, systemic chemotherapy can help alleviate clinical symptoms, reduce or stop tumor growth, and reduce tumor volume. […] MIBG is transported into the cell via the norepinephrine transporter, and ionizing radiation supplied by decaying 131I radionuclides causes cell death. […] Sunitinib is a tyrosine kinase inhibitor that blocks angiogenesis by targeting vascular endothelial growth factor receptor. […] Interferon therapy was described in a single-center retrospective study and 14 patients with metastatic PPGL were evaluated.

• #33 Treatment for paraganglioma with stereotactic radiotherapy

  https://www.wjgnet.com/2307-8960/full/v12/i16/2729.htm

  Treatment for paraganglioma with stereotactic radiotherapy. […] To evaluate the local control and effectiveness of exclusive fractionated stereotactic RT (FSRT) treatment in unresectable PG (uPG). […] We retrospectively evaluated patients with uPG (medically inoperable or refused SR) treated with FSRT with a Cyberknife System (Accuray Incorporated, Sunnyvale, California). […] Our preliminary results on this small cohort of patients suggest that FSRT could be an effective and safe alternative to SR. […] Treatment with fractionated stereotactic radiation therapy can be an effective option for these lesions, especially in reserving facial nerve function. […] For symptomatic patients, to date, common treatment options are surgical resection (SR), embolization with or without surgical resection, radiation therapy (RT), as definitive, adjuvant, or salvage therapy.

• #34 Treatment for paraganglioma with stereotactic radiotherapy

  https://www.wjgnet.com/2307-8960/full/v12/i16/2729.htm

  When PGs are considered unresectable (uPG), alternative treatments are represented by subtotal resection in association with RT (for residual, recurrent tumors or giant tumors) or RT alone. […] Several studies reported a significant local control with acceptable toxicities in patients who underwent subtotal resection followed by RT or treated with RT only. […] RT treatment can be performed with a Cyberknife System (CK), Gamma Knife System (GKS), and linear accelerator (LINAC)-based stereotactic radiosurgery (SRS) with single or multi-fractions schedules [fractionated stereotactic RT (FSRT)]. […] The aim of our study is to evaluate the local control and effectiveness of exclusive FSRT treatment in uPG. […] The treatment was delivered with a median dose of 21 Gy in 4 fractions using the CK. […] No acute and late toxicities were observed. […] All patients had stable or improved clinical status and LC rates were comparable with literature data.

• #35 Paraganglioma | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/paraganglioma.html

  Most doctors will never see a paraganglioma over the lifetime of their careers. So, treatment at an academic medical center which manages a high volume of paragangliomas is important for the best outcomes. […] Stanford is a global referral center for paraganglioma and is at the forefront of developing new, minimally invasive procedures that treat them. […] Recommendations for treatment depend on the size and location of the tumor, its rate of growth, and details of your overall health. Many paragangliomas do not require immediate treatment. […] Active Surveillance If you have a small tumor that isnt causing symptoms, your doctor may recommend periodic MRI imaging and physical exams without other interventions. […] Radiation Therapy A form of radiation therapy called stereotactic radio surgery or CyberKnife may be recommended. CyberKnife is a robotic system that was developed at Stanford. It delivers precise pulses of high-dose radiation to the target tissue, while limiting exposure to surrounding healthy tissues. Radiation cannot remove the tumor, but it can be very effective at stopping its growth.

• #36 Treatment for paraganglioma with stereotactic radiotherapy

  https://www.wjgnet.com/2307-8960/full/v12/i16/2729.htm

  When PGs are considered unresectable (uPG), alternative treatments are represented by subtotal resection in association with RT (for residual, recurrent tumors or giant tumors) or RT alone. […] Several studies reported a significant local control with acceptable toxicities in patients who underwent subtotal resection followed by RT or treated with RT only. […] RT treatment can be performed with a Cyberknife System (CK), Gamma Knife System (GKS), and linear accelerator (LINAC)-based stereotactic radiosurgery (SRS) with single or multi-fractions schedules [fractionated stereotactic RT (FSRT)]. […] The aim of our study is to evaluate the local control and effectiveness of exclusive FSRT treatment in uPG. […] The treatment was delivered with a median dose of 21 Gy in 4 fractions using the CK. […] No acute and late toxicities were observed. […] All patients had stable or improved clinical status and LC rates were comparable with literature data.

• #37 Paraganglioma of the skull base treated with intensity-modulated radiation therapy | Applied Radiation Oncology

  https://www.appliedradiationoncology.com/articles/paraganglioma-of-the-skull-base-treated-with-intensity-modulated-radiation-therapy

  Overall, radiation therapy and SRS provide equivalent local control compared to surgery, with the benefit of decreased morbidity. […] Nonsurgical candidates or those with high potential for neurovascular morbidity may benefit from radiation therapy, typically IMRT. […] Here we report a case of a skull base/neck paraganglioma that was treated with IMRT and achieved local control with 6 years of follow-up.

• #38 Paraganglioma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/paraganglioma/diagnosis-treatment/drc-20575710

  Targeted therapy uses medicines that attack specific chemicals in tumor cells. By blocking these chemicals, targeted treatments can cause tumor cells to die. For paraganglioma, targeted therapy medicines might be used if surgery isn’t an option. Targeted therapy also may be used if the tumor spreads to other parts of the body. […] Peptide receptor radionuclide therapy, also called PRRT, uses medicine to give radiation directly to tumor cells. The medicine combines a substance that finds tumor cells with a substance that contains radiation. The medicine is given through a vein. The medicine goes through the body and sticks to paraganglioma cells. Over days to weeks, the medicine delivers radiation directly to the tumor cells. […] Clinical trials are studies of new treatments or new ways of using older treatments. If you’re interested in clinical trials for paraganglioma, talk with your healthcare professional about your options. Together you can weigh the benefits and risks of treatments that researchers are studying.

• #39 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  After surgery, the blood or urine are checked to ensure metanephrine levels and subsequently, catecholamine levels have returned to normal. Normal metanephrine levels are a sign that all the paraganglioma cells were removed, if the tumor was secreting catecholamines. […] Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. […] Paraganglioma can be treated with MIBG, which is a radiopharmaceutical that is injected into the patients bloodstream. It travels to and binds to the tumor delivering a targeted high dose of radiation directly to the cancer cells. […] PRRT (Peptide Receptor Radionuclide Therapy) is a radiopharmaceutical therapy that when injected into the patients bloodstream travels to and binds to the tumor delivering a targeted high dose of radiation directly to the cancer cells.

• #40 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  The only officially (FDA)-approved treatment option in the US is high-specific activity (HSA) [131I]MIBG therapy (Ultratrace). […] The best investigated and well established therapy for metastatic PGL/PCC is [131I]MIBG therapy, which is preferentially recommended for slow-growing [123/131I]MIBG positive metastatic PCCs/PGLs, although the studies are very heterogeneous and most are retrospective. […] In a meta-analysis of 17 studies with 243 PCC/PGL patients on [131I]MIBG therapy, there was a complete response in 3% of patients, a partial response in 27% of patients and stable disease in 52% of patients: 40% of patients showed a partial hormonal response. […] In two of these studies, the mean progression-free survival (PFS) of [131I]MIBG treated patients was 23.1 and 28.5 months, respectively.

• #41 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  The only officially (FDA)-approved treatment option in the US is high-specific activity (HSA) [131I]MIBG therapy (Ultratrace). […] The best investigated and well established therapy for metastatic PGL/PCC is [131I]MIBG therapy, which is preferentially recommended for slow-growing [123/131I]MIBG positive metastatic PCCs/PGLs, although the studies are very heterogeneous and most are retrospective. […] In a meta-analysis of 17 studies with 243 PCC/PGL patients on [131I]MIBG therapy, there was a complete response in 3% of patients, a partial response in 27% of patients and stable disease in 52% of patients: 40% of patients showed a partial hormonal response. […] In two of these studies, the mean progression-free survival (PFS) of [131I]MIBG treated patients was 23.1 and 28.5 months, respectively.

• #42 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  Very recently, high-specific activity (HSA) [131I]MIBG that consists almost entirely of 131I-labeled molecules has been developed. […] A recent multicentric phase II study led to the FDA-approval of HSA [131I]MIBG (Ultratrace, Azedra) in the United States. […] Of the 68 patients who received at least one therapeutic dose of HSA [131I]MIBG, 17 (25%) had a durable reduction in baseline anti-hypertensive medication use. […] Of the 64 patients with evaluable disease, most patients (59/64, 92%) showed partial responses (23%, 15/64) or stable disease (44/64, 69%) as the best objective response. […] The median OS was 36.7 months (95% confidence interval, 29.9–49.1 months) with 18 months for patients who received one therapeutic dose and 44 months for those who received two therapeutic doses.

• #43 Paraganglioma: Symptoms, Causes and Treatments

  https://www.cancercenter.com/cancer-types/neuroendocrine-tumors/types/paraganglioma

  If the paraganglioma is not causing symptoms, the care team may recommend a watch-and-wait approach. Many paragangliomas grow very slowly, so doctors will check regularly to ensure that the patient’s condition hasnt changed drastically. This is called watchful waiting. […] A paraganglioma that has turned cancerous and metastasized is a challenge. Treatment options are limited. The care team may try to treat metastatic paragangliomas by removing metastases surgically, giving chemotherapy to fight cancer, or using targeted radiotherapies to treat the paragangliomas. […] A newer technique for paraganglioma care involves the use of theranostics. This technology pairs diagnostic biomarkers with therapeutic agents to identify and target cancer cells for destruction. […] The Food and Drug Administration has approved a drug called Azedra (I131-iobenguane/MIBG) to treat patients 12 years and older whove been diagnosed with local, advanced or metastatic pheochromocytomas and paragangliomas. […] After treatment for a paraganglioma, the patient will likely need yearly testing to check for elevated hormone levels from a cancer recurrence.

• #44 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  After surgery, the blood or urine are checked to ensure metanephrine levels and subsequently, catecholamine levels have returned to normal. Normal metanephrine levels are a sign that all the paraganglioma cells were removed, if the tumor was secreting catecholamines. […] Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. […] Paraganglioma can be treated with MIBG, which is a radiopharmaceutical that is injected into the patients bloodstream. It travels to and binds to the tumor delivering a targeted high dose of radiation directly to the cancer cells. […] PRRT (Peptide Receptor Radionuclide Therapy) is a radiopharmaceutical therapy that when injected into the patients bloodstream travels to and binds to the tumor delivering a targeted high dose of radiation directly to the cancer cells.

• #45 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  The most common treatment-related side effects were nausea, myelosuppression, and fatigue, with a higher rate of hematologic toxicity compared to conventional [131I]MIBG-therapy. […] Since most PCCs/PGLs strongly express somatostatin receptor subtype 2 (SSTR2), PRRT using radiolabeled somatostatin analogs has been studied in several small studies and recently reviewed. […] Therefore, latterly, PRRT—a treatment option that has already been approved for gastro-enteropancreatic and pulmonary neuroendocrine tumors in many countries—has also been suggested as an effective treatment option for metastatic PCCs/PGLs. […] PRRT planning is always preceded by somatostatin receptor imaging as part of a theranostic approach. […] Although most studies consist of small numbers and limited follow-up, in one direct comparison study the percentage of patients with tumors showing disease stabilization was significantly greater after PRRT using [177Lu]Lu-DOTA(Tyr3)octreotate (TATE) compared to [131I]MIBG, with a longer PFS/OS of 38.5/60.8 months in the [177Lu]Lu-DOTATATE group versus 20.6/41.2 months in the [131I]MIBG group.

• #46 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  The most common treatment-related side effects were nausea, myelosuppression, and fatigue, with a higher rate of hematologic toxicity compared to conventional [131I]MIBG-therapy. […] Since most PCCs/PGLs strongly express somatostatin receptor subtype 2 (SSTR2), PRRT using radiolabeled somatostatin analogs has been studied in several small studies and recently reviewed. […] Therefore, latterly, PRRT—a treatment option that has already been approved for gastro-enteropancreatic and pulmonary neuroendocrine tumors in many countries—has also been suggested as an effective treatment option for metastatic PCCs/PGLs. […] PRRT planning is always preceded by somatostatin receptor imaging as part of a theranostic approach. […] Although most studies consist of small numbers and limited follow-up, in one direct comparison study the percentage of patients with tumors showing disease stabilization was significantly greater after PRRT using [177Lu]Lu-DOTA(Tyr3)octreotate (TATE) compared to [131I]MIBG, with a longer PFS/OS of 38.5/60.8 months in the [177Lu]Lu-DOTATATE group versus 20.6/41.2 months in the [131I]MIBG group.

• #47 Paraganglioma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/22394-paraganglioma

  How is paraganglioma treated? Treatment options for paraganglioma depend on several factors, including: […] If you have a paraganglioma that causes symptoms due to excess adrenal hormones, your healthcare provider will likely recommend medication to manage the symptoms. Medications may include: […] Treatment options for paraganglioma include: […] Surgery is the main form of treatment for paragangliomas. During the surgery to remove the tumor, your surgeon will check the surrounding tissue and lymph nodes to see if the tumor has spread. […] Radiation therapy is a cancer treatment that focuses strong beams of energy to destroy cancer cells or keep them from growing while sparing as much surrounding healthy tissue as possible. […] Chemotherapy is the standard therapy for treating metastatic paraganglioma. […] Ablation therapy is a minimally invasive treatment option that uses very high or very low temperatures to destroy tumors. […] Targeted therapy is a treatment option that uses medications or other substances to attack specific cancer cells without harming healthy cells.

• #48 The Management of Phaeochromocytomas and Paragangliomas in the Era of Precision Medicine: Where Are We Now? Evidence-Based Systemic Treatment Options and Future Cluster Oriented Perspectives

  https://www.mdpi.com/1424-8247/17/3/354

  In advanced and metastatic setting, surgical resection of the primary tumor or metastases might be considered cautiously on a case-by-case basis, as it may improve symptoms related to CMN secretion or tumor mass effects. However, the impact of surgical removal of the primary tumor in metastatic disease on Overall survival (OS) still remains controversial, although recent studies suggested that it may improve OS. […] The management of metastatic PPGLs is challenging as there are only practiced standards of systemic therapy, that have been extensively adopted outside of controlled clinical trials. To date, the only FDA-approved treatment option in metastatic disease is HSA 131 MIBG in the US. […] Chemotherapy should be considered as the first line treatment in patients who have no significant uptake of radiotracers or with clear rapid disease progression, associated with high tumor burden, or with severe uncontrolled symptoms due to CMNs secretion or mass effect, in order to obtain disease control and/or symptom palliation.

• #49 Paraganglioma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/paraganglioma/diagnosis-treatment/drc-20575710

  Surgery can be done to remove a paraganglioma. Even if a paraganglioma can’t be fully removed, your healthcare professional may recommend surgery to remove as much of the tumor as possible. […] Radiation therapy uses powerful energy beams to treat tumors. The energy can come from X-rays, protons or other sources. Your healthcare professional may recommend radiation if your paraganglioma can’t be removed completely with surgery. Radiation also can help ease pain caused by a paraganglioma that spreads to other parts of the body. […] Ablation therapy uses heat or cold to kill tumor cells and control the growth of the paraganglioma. This may be an option if a paraganglioma has spread to other parts of the body. […] Chemotherapy is a treatment that uses strong medicines. If your paraganglioma has spread, your healthcare professional may recommend chemotherapy to help shrink the tumors. If your paraganglioma makes extra hormones, you receive medicines to control the hormone levels before starting chemotherapy.

• #50 Systemic Radiopharmaceutical Therapy of Pheochromocytoma and Paraganglioma | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/62/9/1192

  Whereas benign pheochromocytomas and paragangliomas are often successfully cured by surgical resection, treatment of metastatic disease can be challenging in terms of both disease control and symptom control. Fortunately, several options are available, including chemotherapy, radiation therapy, and surgical debulking. […] The goals of treatment are palliative, including prevention of progression and improvement of symptoms related to tumor burden or catecholamine release. For systemic treatment, the most common chemotherapy regimen is cisplatin, vinblastine, and dacarbazine, which may be preferred for rapidly progressing disease. […] Currently, systemic radiopharmaceutical therapies (RPT) for pheochromocytoma and paraganglioma include 131I-metaiodobenzylguanidine (iobenguane, 131I-MIBG) of low specific activity (LSA) or high specific activity (HSA) and peptide receptor radionuclide therapy (PRRT) with 90Y- or 177Lu-DOTA-somatostatin analogs.

• #51 Childhood Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Women’s Health – Associates for Women’s Medicine – Syracuse NY Gynecologist, Gynecology, Obstetrics, OBGYN, OB Physicians, Syracuse New York

  https://www.afwomensmed.com/health-library/hw-view.php?DOCHWID=ncicdr0000800108

  Treatment options for childhood paraganglioma and pheochromocytoma include the following: […] Treatment of paraganglioma and pheochromocytoma is surgical. For secreting tumors, alpha- and beta-adrenergic blockade must be optimized before surgery. […] For patients with metastatic disease, responses have been documented to some chemotherapeutic regimens such as gemcitabine and docetaxel or different combinations of vincristine, cyclophosphamide, doxorubicin, and dacarbazine. […] Responses have also been obtained with high-dose 131I-MIBG and sunitinib. […] Specific consensus guidelines for the diagnosis and management of pheochromocytoma and paraganglioma in patients with germline SDHB and SDHD pathogenic variants have been published.

• #52 Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Skin Center

  https://www.skincenter.com/patient-education/healthwise?DOCHWID=ncicdr0000681246

  If all identifiable disease is resectable, including a limited number of distant metastases, surgery can provide occasional long-term remission. […] Chemotherapy has not been shown to improve survival in patients with metastatic pheochromocytoma; however, chemotherapy may be useful for symptom palliation. […] Other palliative treatment modalities include external-beam radiation therapy and embolization, radiofrequency ablation, or cryoablation. […] Treatment options for recurrent pheochromocytoma include: Surgery, Palliative therapy. […] After resection of a localized pheochromocytoma presumed to represent a benign tumor and documented normal postoperative biochemical testing, disease recurrence occurs in 6.5% to 16.5% of patients, and 50% of patients with disease recurrence develop metastatic disease.

• #53 Paraganglioma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/paraganglioma/diagnosis-treatment/drc-20575710

  Targeted therapy uses medicines that attack specific chemicals in tumor cells. By blocking these chemicals, targeted treatments can cause tumor cells to die. For paraganglioma, targeted therapy medicines might be used if surgery isn’t an option. Targeted therapy also may be used if the tumor spreads to other parts of the body. […] Peptide receptor radionuclide therapy, also called PRRT, uses medicine to give radiation directly to tumor cells. The medicine combines a substance that finds tumor cells with a substance that contains radiation. The medicine is given through a vein. The medicine goes through the body and sticks to paraganglioma cells. Over days to weeks, the medicine delivers radiation directly to the tumor cells. […] Clinical trials are studies of new treatments or new ways of using older treatments. If you’re interested in clinical trials for paraganglioma, talk with your healthcare professional about your options. Together you can weigh the benefits and risks of treatments that researchers are studying.

• #54 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  Further clinically controlled studies should be awaited with regard to recommendations and guidelines, including the selection of the radionuclide, doses and dose regimens, but also the consideration of specific risk constellations (nephrotoxicity, patient age, co-medication with SSA). […] In the case of progression after chemotherapy or radionuclide therapy, or if chemotherapy or radionuclide therapy are not possible/tolerated by the patient, the TKIs sunitinib or cabozantinib may be considered, especially for RET and SDHx-mutation carriers. […] Several targeted therapies (PI3K inhibitors in combination with mTORC1 inhibitors, HIF-2α inhibitors, PARP inhibitors, SSTR2 analogs, HDAC inhibitors, DNA demethylating agents) and immunotherapy are currently under investigation and may have strong potential for future personalized therapy approaches.

• #55 A New Approach to the Treatment of Metastatic Paraganglioma: Sorafenib

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4206070/

  Paragangliomas are relatively rare chromaffin cell tumors which may be cured through resection. […] There is no consensus regarding refractory chemotherapy for treatment of metastatic disease. […] Chemotherapy was administered, and no improvement was observed. Therefore, following this palliative conventional chemotherapy, sorafenib was administered for three months, and, finally, positron emission tomography showed that the patients lesions had completely regressed. […] Options for treatment of mPGLs include palliative tumor resection, ablation, reduction of tumor burden and excess of catecholamine related symptoms, and cytotoxic hemotherapy or targeted agents. […] Sorafenib, a mTKI, is used in treatment of advanced renal cell and hepatocellular carcinomas. It is an inhibitor of RAF kinase, c-KIT, FLT-3, RET, VEGFRs (VEGFR-2, VEGFR-3), and PDGFR (PDGFR-). It reduces activation of MEK and MAP kinase, and causes inhibition of cell proliferation, invasion, migration, and tumor growth.

• #56 A New Approach to the Treatment of Metastatic Paraganglioma: Sorafenib

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4206070/

  In our case, the patient obtained clinical and laboratory benefits from sorafenib by showing a truly complete response after a three-month therapy. […] Sorafenib may have potential for treatment of mPGLs since it targets several cellular pathways, one of which is VEGFR-2, -3, and possibly others. […] To the best of our knowledge, in the literature, there are no cases with mPGLs subjected to treatment utilizing sorafenib. Because the options for treatment of mPGLs are rather limited, sorafenib may prove to be a very effective agent.

• #57 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  Somatostatin is a naturally occurring hormone that acts by binding to somatostatin receptor (SSTR), a receptor that is overexpressed in pheo para. SSAs such as octreotide and lanreotide work by activating SSTRs, which can slow tumor growth. […] Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. […] Ablation is a treatment to remove or destroy a body part or tissue or its function. […] Embolization therapy is a treatment to block the artery leading to the adrenal gland. Blocking the flow of blood to the adrenal glands helps kill cancer cells growing there. […] TKI is a targeted therapy treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. […] Treatment of metastatic para can include surgery, MIBG therapy, PRRT therapy (Lutathera), chemotherapy, and others.

• #58 Somatostatin analogues in the treatment of metastatic paraganglioma | ECE2022 | European Congress of Endocrinology 2022 | Endocrine Abstracts

  https://www.endocrine-abstracts.org/ea/0081/ea0081ep575

  Somatostatin analogues in the treatment of metastatic paraganglioma […] Treatment options include surgical resection, chemotherapy, radiotherapy, and 123I-MIBG. […] To date, metastatic PGL can only be cured by surgical resection. Chemotherapy, radiotherapy and 123I-MIBG are used for disease control. Most recently, peptide receptor radionuclide therapy has shown promising results. Somatostatin analogues such as lanreotide are currently used on gastroenteropancreatic neuroendocrine tumors but its role in PGL treatment is still unclear. This case shows an extraordinary response of MPGL to lanreotide, raising interest in this potential therapeutic weapon for a subset of patients who have still few treatment modalities available.

• #59 Paraganglioma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/paraganglioma/diagnosis-treatment/drc-20575710

  Surgery can be done to remove a paraganglioma. Even if a paraganglioma can’t be fully removed, your healthcare professional may recommend surgery to remove as much of the tumor as possible. […] Radiation therapy uses powerful energy beams to treat tumors. The energy can come from X-rays, protons or other sources. Your healthcare professional may recommend radiation if your paraganglioma can’t be removed completely with surgery. Radiation also can help ease pain caused by a paraganglioma that spreads to other parts of the body. […] Ablation therapy uses heat or cold to kill tumor cells and control the growth of the paraganglioma. This may be an option if a paraganglioma has spread to other parts of the body. […] Chemotherapy is a treatment that uses strong medicines. If your paraganglioma has spread, your healthcare professional may recommend chemotherapy to help shrink the tumors. If your paraganglioma makes extra hormones, you receive medicines to control the hormone levels before starting chemotherapy.

• #60 Progress in the diagnosis and treatment of paraganglioma – Wang – Translational Cancer Research

  https://tcr.amegroups.org/article/view/32832/html

  Since the beginning of the 21st century, extracorporeal radiation (EBRT) has been increasingly used in PGL treatment. […] For patients with advanced or unresectable metastatic PGL, radiation therapy can effectively improve symptoms and control tumor growth. […] Interventional radiology techniques for local non-surgical treatment of metastatic PGL include radiofrequency ablation (RFA), cryoablation (CRYO), and percutaneous ethanol injection (PEI). […] For patients with nonresectable, quickly progressing metastatic PPGL, systemic chemotherapy can help alleviate clinical symptoms, reduce or stop tumor growth, and reduce tumor volume. […] MIBG is transported into the cell via the norepinephrine transporter, and ionizing radiation supplied by decaying 131I radionuclides causes cell death. […] Sunitinib is a tyrosine kinase inhibitor that blocks angiogenesis by targeting vascular endothelial growth factor receptor. […] Interferon therapy was described in a single-center retrospective study and 14 patients with metastatic PPGL were evaluated.

• #61 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  In addition, complete metastatic surgery may be considered in oligo-metastatic PCC/PGL on a case-by-case decision, although there is only little evidence for such an approach from single case reports. […] Watchful waiting with frequent follow-up may be the optimal initial approach in patients with non-functioning HN PGL, especially without evidence of significant tumor growth and/or compression of surrounding structures. […] Conventional external beam radiation therapy (cEBRT) or radiosurgery (gamma-knife/cyberknife) are well-established methods in the case of bone metastases and also may play a significant palliative role in oligo-metastatic scenarios. […] Minimally-invasive procedures such as radiofrequency ablation, cryoablation, and ethanol injection may be considered in the treatment of metastatic PCC/PGL, especially in oligo-metastatic disease.

• #62 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  Somatostatin is a naturally occurring hormone that acts by binding to somatostatin receptor (SSTR), a receptor that is overexpressed in pheo para. SSAs such as octreotide and lanreotide work by activating SSTRs, which can slow tumor growth. […] Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. […] Ablation is a treatment to remove or destroy a body part or tissue or its function. […] Embolization therapy is a treatment to block the artery leading to the adrenal gland. Blocking the flow of blood to the adrenal glands helps kill cancer cells growing there. […] TKI is a targeted therapy treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. […] Treatment of metastatic para can include surgery, MIBG therapy, PRRT therapy (Lutathera), chemotherapy, and others.

• #63 Paragangliomas – Treatment, Types & Symptoms | Mount Sinai – New York

  https://www.mountsinai.org/locations/cerebrovascular-center/conditions/tumors/paragangliomas

  At Mount Sinai, we take a multispecialty medical approach to treating paragangliomas. […] At Mount Sinai, we use embolization to treat paragangliomas is embolization. This state-of-the-art technique can shrink the tumor and reduce bleeding, which makes it much easier to remove surgically. We also use angiography to provide information on the precise size and location of the tumors. […] When the tumor is growing slowly and we cannot perform surgery and embolization, we may perform radiation.

• #64 Paraganglioma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/paraganglioma/diagnosis-treatment/drc-20575710

  Sometimes, healthcare professionals recommend against starting paraganglioma treatment right away. Instead, they may prefer to closely watch your condition with regular healthcare checkups. This is known as watchful waiting. For example, watchful waiting may be an option if a paraganglioma grows slowly and doesn’t cause symptoms.

• #65 Paraganglioma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/paraganglioma/diagnosis-treatment/drc-20575710

  Sometimes, healthcare professionals recommend against starting paraganglioma treatment right away. Instead, they may prefer to closely watch your condition with regular healthcare checkups. This is known as watchful waiting. For example, watchful waiting may be an option if a paraganglioma grows slowly and doesn’t cause symptoms.

• #66 Paraganglioma: Symptoms, Causes and Treatments

  https://www.cancercenter.com/cancer-types/neuroendocrine-tumors/types/paraganglioma

  If the paraganglioma is not causing symptoms, the care team may recommend a watch-and-wait approach. Many paragangliomas grow very slowly, so doctors will check regularly to ensure that the patient’s condition hasnt changed drastically. This is called watchful waiting. […] A paraganglioma that has turned cancerous and metastasized is a challenge. Treatment options are limited. The care team may try to treat metastatic paragangliomas by removing metastases surgically, giving chemotherapy to fight cancer, or using targeted radiotherapies to treat the paragangliomas. […] A newer technique for paraganglioma care involves the use of theranostics. This technology pairs diagnostic biomarkers with therapeutic agents to identify and target cancer cells for destruction. […] The Food and Drug Administration has approved a drug called Azedra (I131-iobenguane/MIBG) to treat patients 12 years and older whove been diagnosed with local, advanced or metastatic pheochromocytomas and paragangliomas. […] After treatment for a paraganglioma, the patient will likely need yearly testing to check for elevated hormone levels from a cancer recurrence.

• #67 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  In addition, complete metastatic surgery may be considered in oligo-metastatic PCC/PGL on a case-by-case decision, although there is only little evidence for such an approach from single case reports. […] Watchful waiting with frequent follow-up may be the optimal initial approach in patients with non-functioning HN PGL, especially without evidence of significant tumor growth and/or compression of surrounding structures. […] Conventional external beam radiation therapy (cEBRT) or radiosurgery (gamma-knife/cyberknife) are well-established methods in the case of bone metastases and also may play a significant palliative role in oligo-metastatic scenarios. […] Minimally-invasive procedures such as radiofrequency ablation, cryoablation, and ethanol injection may be considered in the treatment of metastatic PCC/PGL, especially in oligo-metastatic disease.

• #68 Paraganglioma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/paraganglioma/diagnosis-treatment/drc-20575710

  Targeted therapy uses medicines that attack specific chemicals in tumor cells. By blocking these chemicals, targeted treatments can cause tumor cells to die. For paraganglioma, targeted therapy medicines might be used if surgery isn’t an option. Targeted therapy also may be used if the tumor spreads to other parts of the body. […] Peptide receptor radionuclide therapy, also called PRRT, uses medicine to give radiation directly to tumor cells. The medicine combines a substance that finds tumor cells with a substance that contains radiation. The medicine is given through a vein. The medicine goes through the body and sticks to paraganglioma cells. Over days to weeks, the medicine delivers radiation directly to the tumor cells. […] Clinical trials are studies of new treatments or new ways of using older treatments. If you’re interested in clinical trials for paraganglioma, talk with your healthcare professional about your options. Together you can weigh the benefits and risks of treatments that researchers are studying.

• #69 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  Further clinically controlled studies should be awaited with regard to recommendations and guidelines, including the selection of the radionuclide, doses and dose regimens, but also the consideration of specific risk constellations (nephrotoxicity, patient age, co-medication with SSA). […] In the case of progression after chemotherapy or radionuclide therapy, or if chemotherapy or radionuclide therapy are not possible/tolerated by the patient, the TKIs sunitinib or cabozantinib may be considered, especially for RET and SDHx-mutation carriers. […] Several targeted therapies (PI3K inhibitors in combination with mTORC1 inhibitors, HIF-2α inhibitors, PARP inhibitors, SSTR2 analogs, HDAC inhibitors, DNA demethylating agents) and immunotherapy are currently under investigation and may have strong potential for future personalized therapy approaches.

• #70 Childhood Pheochromocytoma & Paraganglioma Treatment – NCI

  https://www.cancer.gov/types/pheochromocytoma/patient/child-pheochromocytoma-treatment-pdq

  There are different types of treatment for children with pheochromocytoma or paraganglioma. […] Children with pheochromocytoma or paraganglioma should have their treatment planned by a team of doctors who are experts in treating childhood cancer. […] Four types of standard treatment are used: Surgery, Chemotherapy, High-dose 131I-MIBG therapy, Targeted therapy. […] Treatment of pheochromocytoma and paraganglioma may cause side effects. […] Patients may want to think about taking part in a clinical trial. […] Treatment of newly diagnosed pheochromocytoma and paraganglioma in children may include the following: Surgery to completely remove the tumor, Combination chemotherapy, high-dose 131I-MIBG therapy, or targeted therapy for tumors that have spread to other parts of the body. […] Before surgery, drug therapy with alpha-blockers to control blood pressure and beta-blockers to control heart rate are given. […] Treatment of recurrent pheochromocytoma and paraganglioma may include the following: A clinical trial that checks a sample of the patient’s tumor for certain gene changes. […] A clinical trial of 131I-MIBG therapy. […] A clinical trial of a new chemotherapy drug (guadecitabine).

• #71 Pheochromocytoma | UCSF Department of Surgery

  https://endocrinesurgery.ucsf.edu/condition/pheochromocytoma

  Treatment for inherited pheochromocytoma that forms in one adrenal gland is surgery to completely remove the gland. […] Treatment of pheochromocytoma or paraganglioma that has spread to nearby organs or lymph nodes is surgery to completely remove the tumor. […] Treatment of metastatic pheochromocytoma or paraganglioma may include: Surgery to completely remove the cancer, including tumors that have spread to distant parts of the body. […] Treatment of recurrent pheochromocytoma or paraganglioma may include: Surgery to completely remove the cancer. […] Treatment of pregnant women with pheochromocytoma may include surgery.

• #72 Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Statesboro Ear, Nose, & Throat Clinic

  https://drcrews.com/patient-education/healthwise?DOCHWID=ncicdr0000681246

  Phenoxybenzamine use is safe in pregnancy, but beta-adrenergic blockers should be initiated only if needed because their use has been associated with intrauterine growth restriction. […] Resection of the tumor can often be performed safely during the second trimester, or tumor resection can be combined with cesarean delivery for patients diagnosed later in pregnancy.

• #73

  http://www.cancerhelpessentiahealth.org/Cancer_Types/pheochromocytoma_277407E2_05.html

  Treatment of metastatic pheochromocytoma or paraganglioma may include the following: Surgery to completely remove the cancer, including tumors that have spread to distant parts of the body. Palliative therapy, to relieve symptoms and improve the quality of life, including: Surgery to remove as much cancer as possible. Combination chemotherapy. Radiation therapy with 131I-MIBG. External radiation therapy to areas (such as bone) where cancer has spread and cannot be removed by surgery. Embolization (treatment to block an artery that supplies blood to a tumor). Ablation therapy using radiofrequency ablation or cryoablation for tumors in the liver or bone. A clinical trial of targeted therapy with a tyrosine kinase inhibitor. A clinical trial of internal radiation therapy using a new radioactive substance.

• #74

  http://www.cancerhelpessentiahealth.org/Cancer_Types/pheochromocytoma_277407E2_05.html

  Treatment of metastatic pheochromocytoma or paraganglioma may include the following: Surgery to completely remove the cancer, including tumors that have spread to distant parts of the body. Palliative therapy, to relieve symptoms and improve the quality of life, including: Surgery to remove as much cancer as possible. Combination chemotherapy. Radiation therapy with 131I-MIBG. External radiation therapy to areas (such as bone) where cancer has spread and cannot be removed by surgery. Embolization (treatment to block an artery that supplies blood to a tumor). Ablation therapy using radiofrequency ablation or cryoablation for tumors in the liver or bone. A clinical trial of targeted therapy with a tyrosine kinase inhibitor. A clinical trial of internal radiation therapy using a new radioactive substance.

• #75 Pheochromocytoma | UCSF Department of Surgery

  https://endocrinesurgery.ucsf.edu/condition/pheochromocytoma

  Pheochromocytoma and paraganglioma are rare tumors that come from the same type of tissue. […] Genetic counseling is part of the treatment plan for patients with pheochromocytoma or paraganglioma. […] There are different types of treatment for patients with pheochromocytoma or paraganglioma. […] Patients receive medication to treat the signs and symptoms of pheochromocytoma and paraganglioma. […] The following types of treatment are used: Surgery, Radiation therapy, Chemotherapy, Ablation therapy, Embolization therapy, Targeted therapy. […] New types of treatment are being tested in clinical trials. […] Treatment for pheochromocytoma and paraganglioma may cause side effects. […] Follow-up care will be needed. […] Treatment of localized benign pheochromocytoma or paraganglioma is usually surgery to completely remove the tumor.

• #76 Paraganglioma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/paraganglioma

  After surgery, the blood or urine are checked to ensure metanephrine levels and subsequently, catecholamine levels have returned to normal. Normal metanephrine levels are a sign that all the paraganglioma cells were removed, if the tumor was secreting catecholamines. […] Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. […] Paraganglioma can be treated with MIBG, which is a radiopharmaceutical that is injected into the patients bloodstream. It travels to and binds to the tumor delivering a targeted high dose of radiation directly to the cancer cells. […] PRRT (Peptide Receptor Radionuclide Therapy) is a radiopharmaceutical therapy that when injected into the patients bloodstream travels to and binds to the tumor delivering a targeted high dose of radiation directly to the cancer cells.

• #77 Pheochromocytoma and Paraganglioma: From Treatment to Follow-up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7751245/

  Pheochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors arising from chromaffin cells in the adrenal medulla, sympathetic or parasympathetic ganglia. Currently, the only curative treatment option of pheochromocytomas/paraganglioma (PPGL) is surgical resection. Surgery aims to eliminate both risks of hypersecretion and tumor growth. The consequences of hypersecretion should be carefully controlled with medical therapy before and during the surgery. […] Therefore, all patients with PPGL who are surgically treated should be followed for at least 10 years for recurrent disease and new tumor formation. […] Although surgical resection is the only curative treatment for PPGLs, surgical treatment is palliative except for resectable locoregional metastases in metastatic disease or for isolated distant metastases. The purpose of palliative treatment is to reduce hormone secretion and prevent metastasis-related complications in a critical anatomical location.

• #78 Pheochromocytoma and Paraganglioma: From Treatment to Follow-up

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7751245/

  If curative surgical resection cannot be performed, cytoreductive surgery should be considered to decrease the volume of the target tumor tissue and increase the effectiveness of other therapeutic options, such as targeted radiotherapy. […] Since all patients with PPGL have a risk of recurrence, they should be followed up after R0 resection. […] Lifelong follow-up is recommended for high-risk PCC patients (germline mutation, young age (20 years), large tumor (56 cm), SDHB carriers) and all PGL patients.

• #79 The Management of Phaeochromocytomas and Paragangliomas in the Era of Precision Medicine: Where Are We Now? Evidence-Based Systemic Treatment Options and Future Cluster Oriented Perspectives

  https://www.mdpi.com/1424-8247/17/3/354

  The choice of the first line treatment and of subsequent therapy approaches depends on the patients’ characteristics, clinical symptoms, tumor type, radioisotope tumor avidity, disease burden (limited/extensive) as well as its aggressivity (limited stable disease, extensive disease with slow/rapid progression) and available resources. […] A multidisciplinary approach by an expert medical team is needed in order to define the most proper, personalized treatment plan based on both patient characteristics (age, health status) and tumour features (location, size, extent, biochemical profile, functional imaging and eventually genetic background), weighing the pros and cons of each option.

• #80 The Management of Phaeochromocytomas and Paragangliomas in the Era of Precision Medicine: Where Are We Now? Evidence-Based Systemic Treatment Options and Future Cluster Oriented Perspectives

  https://www.mdpi.com/1424-8247/17/3/354

  The choice of the first line treatment and of subsequent therapy approaches depends on the patients’ characteristics, clinical symptoms, tumor type, radioisotope tumor avidity, disease burden (limited/extensive) as well as its aggressivity (limited stable disease, extensive disease with slow/rapid progression) and available resources. […] A multidisciplinary approach by an expert medical team is needed in order to define the most proper, personalized treatment plan based on both patient characteristics (age, health status) and tumour features (location, size, extent, biochemical profile, functional imaging and eventually genetic background), weighing the pros and cons of each option.

• #81 The Management of Phaeochromocytomas and Paragangliomas in the Era of Precision Medicine: Where Are We Now? Evidence-Based Systemic Treatment Options and Future Cluster Oriented Perspectives

  https://www.mdpi.com/1424-8247/17/3/354

  Pheochromocytomas (PCCs) and Paragangliomas (PGLs), commonly known as PPGLs to include both entities, are rare neuroendocrine tumors that may arise in the context of hereditary syndromes or be sporadic. However, even among sporadic PPGLs, identifiable somatic alterations in at least one of the known susceptibility genes can be detected. Therefore, about 3/4 of all PPGL patients can be assigned to one of the three molecular clusters that have been identified in the last years with difference in the underlying pathogenetic mechanisms, biochemical phenotype, metastatic potential, and prognosis. While surgery represents the mainstay of treatment for localized PPGLs, several therapeutic options are available in advanced and/or metastatic setting. However, only few of them hinge upon prospective data and a cluster-oriented approach has not yet been established. In order to render management even more personalized and improve the prognosis of this molecularly complex disease, it is undoubtable that genetic testing for germline mutations as well as genome profiling for somatic mutations, where available, must be improved and become standard practice. This review summarizes the current evidence regarding diagnosis and treatment of PPGLs, supporting the need of a more cluster-specific approach in clinical practice.

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